Hereditary fructose intolerance in a Gurkha family.

Introduction Hereditary Fructose Intolerance I~FI) began toO appear in ,the literature after Chambers and Pmj)t1 in H56 delscrilbed an aduLt with an idiosyncrasy to fruotose. Froesch, Prader and colleagues2, the following year, described, for .the 'first time the typical syndrome.o'f HP!. Since then, over one hundred patients have been described ['fOm around the world indicruting ,a wide distdbution ,o[ this genetic defect, except Israel. The frequency is estimated as 1:30,000 and it pfOIbaJbly has an autosomal recessiv:e pattern .of inheritance. ilt has been reported more commonly from Europe where ~he ,consumption of Isucrose containing foods ,alppears to be high. The syndrome has never been described among Nepalese children and for this reason, together with ,its rarity, difficulty in diagnosis and management among Gurkhas it was feltwol'thwhile recording Vhis family.